Terapi Suportif Pada Anak Yang Mengalami Thalasemia Mayor: Literature Review

Abstract

Latar belakang: Thalasemia adalah salah satu penyakit anemia hemolitik secara genetik dari orang tua kepada anak yang diturunkan secara resesif, secara klinis dibedakan atas thalasemia mayor dan minor. Angka kejadian thalasemia sampai saat ini masih tinggi, menurut World Health Organization (WHO) 2018, Hampir (7%) penduduk dunia mempunyai gen thalasemia dan di asia sendiri kejadian tertinggi sampai dengan (40%). Diindonesia sendiri hampir setiap kelahiran 3.000 bayi memiliki keturanan thalasemia, dan didukung dari yayasan thalasemia Indonesia ditahun (2012) 4.896 naik menjadi 9.028 ditahun (2018).

Tujuan: Penelitian ini bertujuan untuk menganalisis terapi suportif pada anak yang mengalami thalasemia mayor

Mertode: penelitian ini menggunakan The JBI Critical Appraisal Tools untuk mengkaji risiko bias dalam studi dengan tiga database (Googel scholar, DOAJ, dan Pubmed) Sehingga hasil akhir diperoleh 11 artikel untuk digunakan sebagai tinjauan literature yang siap dianalisis.

Hasil : terdapat hubungan dalam penerapan terapi suportif pada anak dangan thalasemia dimana terapi gen pada anak thalasemia sangat efektif dalam menagani ketergantungan dengan transfusi darah dan komsumsi terapi pemberian kalasi besi. Selanjutnya terdapat hubungan signifikan antara kepatuhan konsumsi kalasi besi setelah transfusi darah dengan pertumbuhan anak thalasemia, lalu penerapan suplemen nutrisi makanan dalam bentuk vitamin E menunjukan bahwa asupan protein dan asupan vitamin E berhubungan dengan kadar hemoglobin anak dengan thalasemia.

Simpulan: pemberian terapi suportif pada anak thalasemia memiliki hubungan yang sigfnikan dalam pemberian terapi sehingga peran orang tua juga mampu memberikan pengobatan dengan alternatife pilihan pengobatan yang baik sehingga dapat diterapkan kepada anak yang mengalami thalasemia.

Kata kunci: Kalasi besi, pertumbuhan anak thalasemia, suplemen nutrisi, terapi suportif, thalasemia, thalasemia mayor.

Background: Thalassemia is a hemolytic anemia disease genetically from parents to children which is inherited recessively, clinically differentiated into thalassemia major and minor. The incidence of thalassemia is still high, according to the World Health Organization (WHO) 2018, Almost (7%) of the world's population has a thalassemia gene and in Asia itself the highest incidence is up to (40%). In Indonesia alone, almost every 3,000 babies born have thalassemia offspring, and supported by the Indonesian Thalassemia Foundation in (2012), 4,896 rose to 9,028 in (2018).

Objective: This study aims to analyze supportive therapy in children with thalassemia major

Method: this study uses The JBI Critical Appraisal Tools to assess the risk of bias in a study with three databases (Googel scholar, DOAJ, and Pubmed) So that the final results obtained 11 articles to be used as a literature review that is ready to be analyzed.

Results: there is a relationship in the application of supportive therapy in children with thalassemia where gene therapy in children with thalassemia is very effective in treating dependence with blood transfusions and consumption of iron calcification therapy. Furthermore, there is a significant relationship between adherence to iron calcium consumption after blood transfusion with the growth of thalassemia children, then the application of dietary nutritional supplements in the form of vitamin E shows that protein intake and vitamin E intake are associated with hemoglobin levels in children with thalassemia.

Conclusion: the provision of supportive therapy in thalassemia children has a significant relationship in the provision of therapy so that the role of parents is also able to provide treatment with good alternative treatment options so that it can be applied to children with thalassemia.

Keywords: Iron calcification, growth of thalassemia children, nutritional supplements, supportive therapy, thalassemia, thalassemia major.